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单次剂量因子给药在接受包皮环切术的严重血友病新生儿中的止血效果。

Haemostatic efficacy of single-dose factor administration in neonates with severe haemophilia undergoing circumcision.

机构信息

Stead Family Department of Pediatrics, University of Iowa, Iowa City, IA, USA.

Carver College of Medicine, University of Iowa, Iowa City, IA, USA.

出版信息

Haemophilia. 2020 Sep;26(5):786-792. doi: 10.1111/hae.14122. Epub 2020 Aug 3.

Abstract

INTRODUCTION

Perioperative management of children with haemophilia undergoing surgery is a complex and understudied topic. Circumcision is the most common procedure performed in the neonatal period, and guidelines to prevent bleeding complications from circumcision are lacking. Treatment protocols vary widely, and many centres treat patients with factor products for up to two weeks after circumcision. There is an unmet need for studies evaluating optimal factor replacement therapy around the time of circumcision in neonates with severe haemophilia.

AIM

To determine the efficacy of a single dose of factor replacement before circumcision to prevent bleeding complications in neonates with severe haemophilia.

METHODS

We conducted a retrospective chart review of male infants born between January 2000 and June 2019. Male neonates with severe haemophilia diagnosed at the Iowa Hemophilia and Thrombosis Center (n = 22) and healthy newborn controls who underwent circumcision at the University of Iowa Hospitals were included. Data were collected from the electronic medical record. Neonates with severe haemophilia were separated into two groups-those pretreated with one dose of factor replacement before circumcision and those without pretreatment.

RESULTS

We observed that neonates with severe haemophilia pretreated with a single dose of factor VIII or factor IX replacement had significantly reduced bleeding complications, shorter hospital stay and required less therapeutic intervention compared with untreated patients. Importantly, pretreated patients had outcomes similar to healthy controls.

CONCLUSIONS

Our results demonstrate that a single dose of factor replacement before circumcision is effective to prevent bleeding in neonates with severe haemophilia.

摘要

引言

患有血友病的儿童在接受手术时的围手术期管理是一个复杂且研究不足的课题。包皮环切术是新生儿期最常见的手术,但缺乏预防该手术出血并发症的指南。治疗方案差异很大,许多中心在新生儿接受包皮环切术之后的两周内都使用因子产品治疗患者。对于严重血友病新生儿在包皮环切术前后的最佳因子替代治疗方案,仍存在未满足的研究需求。

目的

确定在新生儿接受包皮环切术前单次给予因子替代治疗以预防严重血友病患儿出血并发症的疗效。

方法

我们对 2000 年 1 月至 2019 年 6 月期间出生的男性婴儿进行了回顾性病历审查。爱荷华血友病和血栓形成中心(Iowa Hemophilia and Thrombosis Center)诊断为严重血友病的男性新生儿(n=22)和在爱荷华大学医院接受包皮环切术的健康新生儿对照组纳入研究。数据从电子病历中收集。将严重血友病新生儿分为两组——在包皮环切术前接受单次剂量因子替代治疗的组和未接受预处理的组。

结果

我们观察到,与未接受治疗的患者相比,接受单次剂量 VIII 因子或 IX 因子替代治疗的严重血友病新生儿出血并发症明显减少,住院时间缩短,需要的治疗干预也更少。重要的是,预处理患者的结局与健康对照组相似。

结论

我们的结果表明,在新生儿接受包皮环切术前单次给予因子替代治疗可有效预防严重血友病患儿的出血。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c624/11960129/8028ad56ce22/nihms-2067424-f0001.jpg

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